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Rheumatic fever is a systemic disease involving joints, heart, pleura, skin and subcutaneous tissue and basal ganglion(l-4) Jaccoud's Arthritis is a rare manifestation of rheumatoid arthritis involving small joints of hands leading to a prolonged disability and deformity called as Jaccoud’s Arthritis(l)
Rheumatic fever is a systemic disease involving joints, heart, pleura, skin and subcutaneous tissue and basal ganglion(l-4) Jaccoud's Arthritis is a rare manifestation of rheumatoid arthritis involving small joints of hands leading to a prolonged disability and deformity called as Jaccoud’s Arthritis(l)
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Rheumatic fever is a systemic disease involving joints, heart, pleura, skin and subcutaneous tissue and basal ganglion(l-4) Jaccoud's Arthritis is a rare manifestation of rheumatoid arthritis involving small joints of hands leading to a prolonged disability and deformity called as Jaccoud’s Arthritis(l)
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3. Friede RL, Boltshauser E. Uncommon Clementi M, Tencoli R. Chorioretinal
syndromes of cerebellar vermisplasia. coloboma and Joubert syndrome: A non- Dev Med Child Neurol 1978, 20: 758-763. random association. J Pediatr 1984, 105: 282-284. 4. Casamassina AC, Pfeiffer RA. Joubert Syndrome. In: Birth Defects Encyclo- 8. Van Dorp DB, Alzbeta Palan, Mei Lan pedia. Ed Buyse ML. Dover, Blackwell Kwee, Barth PG van der Harten JJ. Scientific Publications, Inc, 1990, pp Joubert Syndrome: A clinical and patho- 995-996. logical description of an affected male and a female fetus from the same sibship. 5. McKusick VA. Joubert Syndrome. In: Am J Med Gen 1991, 40: 100-104. Mendelian Inheritance in Man, 10th edn, Vol 2. Ed McKusick VA. Baltimore, The 9. Kendall B, Kingsley D, Lambert SR, Johns Hopkins University Press, 1992, p Taylor D, Finn P. Joubert Syndrome: A 1272. clinico-radiologic study. Neuroradiology 6. Lindhout D, Barth PG, Valk J, Boen-Tan 1990, 31: 502-506. TN. The Joubert Syndrome associated 10. Campbell S, Tsannatos CH, Pearce JM. with bilateral chorioretinal coloboma. Eur The prenatal diagnosis of Joubert's Syn- J Pediatr 1980, 134: 173-176. drome of familial agenesis of the cerebel- 7. Laverda AM, Saia OS, Drigo P, Danieli E, lar vermis. Prenat Diag 1984, 4: 391-395.
Jaccoud's Arthritis still remains a common disease in develop-
ing countries like India accounting for 30- K.C. Aggarwal 40% of total heart diseases(l,2). We present H. Chellani here a rare manifestation of rheumatic fever R. Mehta Involving small joints of hands leading to a S. Gupta prolonged disability and deformity called as Jaccoud's Arthritis(l). Recognition of this entity is important specially for differentia- Rheumatic fever is a systemic disease tion from JRA (Juvenile Rheumatoid Ar- involving joints, heart, pleura, skin and sub- thritis) as long-term streptococcal prophy- cutaneous tissue and basal ganglion(l-4). laxis will be required for the former. Only Although, the incidence of rheumatic fever an isolated case report of this entity is re- has come down in advanced countries, it ported from India and that too in adults(2). From the Department of Pediatrics, Sanjay Case Report Gandhi Memorial Hospital, Delhi Adminis- tration, Mangolpuri, Delhi 110 093. An 8-year-old child presented to us with Reprint requests: Dr. K.C. Aggarwal, 198, persistent joint swelling in ankle, elbow and Pundrik Vihar, Opp. 'D' Block, Saraswati small joints of hand mainly metacarpopha- Vihar, Delhi 110 034. langeal and proximal interphalangeal joints Received for publication: August 31, 1993; of one month duration. History of sore Accepted: June 27, 1994 throat, palpitation, drug intake, epistaxis,
1418 INDIAN PEDIATRICS VOLUME 31-NOVEMBER 1994
rash and dysuria were denied. Past history Discussion
revealed presence of fever with major joint involvement 2 years back. Swelling lasted Presence of three major criteria, namely, arthritis, carditis and subcutaneous nodules, for 8-10 days and the child was not investi- accompanied by laboratory criteria of high gated during that episode. ASO and ESR, positive CRP and polymor- On clinical examination, in addition to phonuclear leucocytosis confirmed the diag- joint findings, subcutaneous nodules (size 1- nosis of rheumatic fever. However, persis- 1.2 cm) on olecranon processes and exten- tence of small joints arthritis of hands for sor surface of hand were noticed. Heart ex- more than 12 weeks with deformity pre- amination revealed tachycardia and grade 2/ sented an enigma initially. 6 apical systolic murmur. No other systemic abnormality was detected. Presence-of sub- Joints are involved in 70% of initial cutaneous nodule and heart murmur attacks of rheumatic fever(l). The arthritis prompted us to investigate the child for is classifically migratory (flitting) lasting a rheumatic fever also. day or so in one joint and then disappearing and involving the other joints. It classically On investigation, throat swab for J3 involves major joints especially knee joint, streptococci hemolyticus was negative. elbow joint and ankle joint. Temporoman- Hemogram showed polymorphonuclear dibular and stemoclavicular joint involve- leucocytosis with ESR of 80. Mantoux test ment is not described in rheumatic fever. was negative. Rheumatoid factor and ANF Very rarely it has been described in rheu- were also negative. Slit lamp examination matic fever with minor or some deformities of eyes showed no evidence of iritis. ASO of hand and feet that can occur which may titre repeated twice was 381 Todd units. be persistent and are known as Jaccoud's CRP was positive. X-ray chest showed no Arthritis(4,8). The exact pathogenesis of cardiomegaly. ECG showed a heart rate of this condition is not clear. Rarely, this con- 124/minute and PR interval was normal dition has also been described in systemic with no rhythm disturbance. ECHO-cardio- lupus erythematosis(5), urticarial hypocom- graphy: 2D and M Mode showed edema of plementic vasculitis(6), malignancies and in mitral cusp with no other abnormality. X- ulcerative colitis(7). However, this entity rays of joints showed no destruction of bony assumes special importance when associ- surfaces but soft tissue swelling around the ated with valvular involvement and other joints were present. Regional osteoporosis features of rheumatic fever. Joint deformity of small joints of hands were also seen. On follow up, the patient was treated with peni- is reversible and overall prognosis is good. cillin and aspirin in the doses of 100 mg/kg At times it may be associated with tendi- and supportive care. After 12 weeks of hos- nous laxity and elongation of pattelar ten- pitalization, the small joints of hands were don which was missing in our case. Another still swollen and painful inspite of full cov- uncommon manifestation of rheumatic fe- erage with aspirin. On follow up 21/2 months ver is simultaneous involvement of multiple after hospital discharge, the swelling of the joints which was also seen in our case. hands had subsided. There were no subcuta- REFERENCES neous nodules. Echo-cardiography had re- vealed slight thickening of mitral valve 1. Ermond RTD. Rheumatic fever. In: Price cusps without any evidence of regurgitation. Textbook of Medicine, 12th edn. Scott
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RB. London, Oxford University Press, knees in systemic lupus erythematosus.
1982, pp 69-70. Maldonaclo-Cocco-ja Clin Rheumatol 1989,8:416-417. 2. Gupta MS, Mehta L, Malhotra S, et al. Jaccoud's arthritis. J Assoc Phys India 6. Kahn-MF. Jaccoud's arthritis and 1990, 38: 947-948. urticarial hypocomplementic vasculitis. J Rheumatol 1989, 16: 252. 3. Tatarkina ND, Dudidov Al, Liubarskaia DA. Postrheumatic Jaccoud's arthritis. 7. Maher JM, Strosberg JM, Rowley RF, et Reumatologica-Mosk 1989, 1: 70-71. al. Jaccoud's arthropathy and inflamma- tory bowel disease. J Rheumatol 1992, 19: 4. Joao-SR, Afonso MR, Martin Junior L, et 1637-1639. al. Jaccoud's syndrome: A case report. Arq-Bras-Cardioj 1989, 53: 221-223. 8. Biasi-D, Carletto A, Caramaschi P, et al. Jaccoud's postrheumatic arthritis. 5. De-la-sota-M. Jaccoud's arthropatby in Recenti-Prvg-Med 1992, 83: 197-199.
Osteogenesis Imperfecta Type II in variety of biochemical defects in type I
One of a pair of Twins procollagen resulting in disruption of triple helical conformation and procollagen suicide are responsible for the clinical features(2). Some cases are autosomal Girish Gupta recessivtf but many are new dominant muta- Praveen Kumar tions. A large majority of patients die in Anil Narang early neonatal period or infancy but the incidence in general population is kept con- stant as a result of new mutations. It has been estimated that the mutation rate in osteogenesis imperfecta is 4 x 10-5 per gene Osteogenesis imperfecta type II also per generation(3). The occurrence of osteo- called osteogenesis imperfecta congenita or genesis imperfecta in one of a pair of twins Vrolik's disease is a rare connective tissue would be a rare chance association. On ex- disease affecting 1 in 62,000 births(l). A tensive review of literature we could come across only two such reports(4,5). The pur- From the Division of Neonatology, Department of Pediatrics, Postgraduate Institute of Med- pose of this communication is to describe ical Education and Research, Chandigarh our experience of such a case, emphasize 160 012. the inclusion of long bone evaluation in the Reprint requests: Dr. Anil Narang, Additional 'routine' antenatal ultrasound examination Professor, Division of Neonatology, Depart- and raise the management dilemma posed ment of Pediatrics, PGIMER, Chandigarh by such a situation. 160012. Case Report Received for publication: February 18, 1994; Accepted: June 30, 1994 Baby S, second of the twins, female